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Genetics Home Reference: your guide to understanding genetic conditions
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TECTA

Reviewed November 2006

What is the official name of the TECTA gene?

The official name of this gene is “tectorin alpha.”

TECTA is the gene's official symbol. The TECTA gene is also known by other names, listed below.

What is the normal function of the TECTA gene?

The TECTA gene provides instructions for making a protein called alpha-tectorin. This protein is found in the inner ear, as part of a structure called the tectorial membrane. The tectorial membrane helps to convert sound waves to nerve impulses, a critical process for normal hearing.

Alpha-tectorin interacts with other proteins to form the tectorial membrane. Two regions of the alpha-tectorin protein, called the vWFD domain and the zona pellucida domain, are important for protein interactions and assembly of the tectorial membrane.

How are changes in the TECTA gene related to health conditions?

nonsyndromic deafness - caused by mutations in the TECTA gene

Researchers have identified several TECTA gene mutations that cause a form of nonsyndromic deafness (hearing loss without related signs and symptoms affecting other parts of the body) called DFNA8/A12. DFNA8/A12 deafness is inherited in an autosomal dominant manner, which means that one copy of an altered TECTA gene in each cell is sufficient to cause hearing loss. TECTA gene mutations replace one of the protein building blocks (amino acids) used to make alpha-tectorin with an incorrect amino acid. TECTA gene mutations affect the vWFD domain or the zona pellucida domain, which are important regions for interactions with other proteins. If the amino acid replacement alters normal protein interactions, it could disrupt the structure of the tectorial membrane and the conversion of sound to nerve impulses.

Researchers have also identified a few TECTA gene mutations that cause a form of nonsyndromic deafness called DFNB21. DFNB21 deafness is inherited in an autosomal recessive manner, which means two copies of the TECTA gene in each cell are altered. These TECTA gene mutations create a premature stop signal in the instructions for making the alpha-tectorin protein. Although the effect of these mutations is not fully understood, most likely no protein is produced or an abnormally small protein is made. A missing or abnormally small alpha-tectorin protein would disrupt the structure of the tectorial membrane and the conversion of sound to nerve impulses.

Where is the TECTA gene located?

Cytogenetic Location: 11q22-q24

Molecular Location on chromosome 11: base pairs 121,102,665 to 121,191,492

The TECTA gene is located on the long (q) arm of chromosome 11 between positions 22 and 24.

The TECTA gene is located on the long (q) arm of chromosome 11 between positions 22 and 24.

More precisely, the TECTA gene is located from base pair 121,102,665 to base pair 121,191,492 on chromosome 11.

See How do geneticists indicate the location of a gene? (http://ghr.nlm.nih.gov/handbook/howgeneswork/genelocation) in the Handbook.

Where can I find additional information about TECTA?

You and your healthcare professional may find the following resources about TECTA helpful.

You may also be interested in these resources, which are designed for genetics professionals and researchers.

What other names do people use for the TECTA gene or gene products?

  • DFNA8
  • DFNA12
  • DFNB21
  • TECTA_HUMAN

See How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What glossary definitions help with understanding TECTA?

acids ; amino acid ; autosomal ; autosomal dominant ; autosomal recessive ; cell ; domain ; gene ; protein ; recessive ; tectorial membrane

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (http://www.ghr.nlm.nih.gov/glossary).

References

  • Balciuniene J, Dahl N, Jalonen P, Verhoeven K, Van Camp G, Borg E, Pettersson U, Jazin EE. Alpha-tectorin involvement in hearing disabilities: one gene--two phenotypes. Hum Genet. 1999 Sep;105(3):211-6. (http://www.ncbi.nlm.nih.gov/pubmed/10987647?dopt=Abstract)
  • Finsterer J, Fellinger J. Nuclear and mitochondrial genes mutated in nonsyndromic impaired hearing. Int J Pediatr Otorhinolaryngol. 2005 May;69(5):621-47. Review. (http://www.ncbi.nlm.nih.gov/pubmed/15850684?dopt=Abstract)
  • Moreno-Pelayo MA, del Castillo I, Villamar M, Romero L, Hernández-Calvín FJ, Herraiz C, Barberá R, Navas C, Moreno F. A cysteine substitution in the zona pellucida domain of alpha-tectorin results in autosomal dominant, postlingual, progressive, mid frequency hearing loss in a Spanish family. J Med Genet. 2001 May;38(5):E13. (http://www.ncbi.nlm.nih.gov/pubmed/11333869?dopt=Abstract)
  • Naz S, Alasti F, Mowjoodi A, Riazuddin S, Sanati MH, Friedman TB, Griffith AJ, Wilcox ER, Riazuddin S. Distinctive audiometric profile associated with DFNB21 alleles of TECTA. J Med Genet. 2003 May;40(5):360-3. (http://www.ncbi.nlm.nih.gov/pubmed/12746400?dopt=Abstract)
  • NCBI Gene (http://www.ncbi.nlm.nih.gov/gene/7007)
  • OMIM: TECTORIN, ALPHA (http://omim.org/entry/602574)
  • Petersen MB, Willems PJ. Non-syndromic, autosomal-recessive deafness. Clin Genet. 2006 May;69(5):371-92. Review. (http://www.ncbi.nlm.nih.gov/pubmed/16650073?dopt=Abstract)
  • Pfister M, Thiele H, Van Camp G, Fransen E, Apaydin F, Aydin O, Leistenschneider P, Devoto M, Zenner HP, Blin N, Nürnberg P, Ozkarakas H, Kupka S. A genotype-phenotype correlation with gender-effect for hearing impairment caused by TECTA mutations. Cell Physiol Biochem. 2004;14(4-6):369-76. (http://www.ncbi.nlm.nih.gov/pubmed/15319541?dopt=Abstract)

 

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

 
Reviewed: November 2006
Published: July 7, 2014